Bejel

Topic Contents

Bejel

National Organization for Rare Disorders, Inc.

Important It is possible that the main title of the report Bejel is not the name you expected. Please check the
synonyms listing to find the alternate name(s) and
disorder subdivision(s) covered by this report.

Synonyms

Dichuchwa

Endemic Syphilis

Frenga

Njovera

Nonvenereal Syphilis

Siti

Treponematosis, Bejel Type

Disorder Subdivisions

None

Related Disorders List

Information on the following diseases can be found in the
Related Disorders section of this report:

Acquired Syphilis

Pinta

Yaws

General Discussion

Bejel is an infectious disease that is rare in the United States but common in certain parts of the world. The infection is very similar to syphilis but is not sexually transmitted. Most frequently, transmission is by means of non-sexual skin contact or by common use of eating and drinking utensils. The organism that causes bejel belongs to the same family as the bacterium that causes syphilis, pinta and yaws and is known as treponema. Treponemas are spiral-shaped bacteria (spirochetes). Also known as endemic syphilis, bejel is characterized by lesions of the skin and bones that begin in the mouth and progress in gradual stages. The late stages are the most severe.

Bejel is very common in dry, hot climates especially in the countries of the eastern Mediterranean region and in Saharan West Africa.

Symptoms

At the outset, children with bejel have patchy, slimy, ulcerations (lesions) on the mucous membranes, particularly in or near the mouth. Later, blisters appear on the back, arms, and legs. Further disease progression is indicated by infections of the bones, especially those of the legs. In these later stages, soft, gummy lumps (gummas) appear on the roof of the mouth and in the nasal passages.

Other symptoms may include swollen glands or lymph nodes (lymph-adenopathy), and/or skin ulcerations under the arms or around the rectum and groin.

Causes

Bejel is a rare infectious disease caused by a spiral-shaped bacterium (spirochete), known as Treponema pallidum endemicum. Under a microscope, T. p. endemicum is virtually indistinguishable from Treponema pallidum, the bacterium that causes syphilis. The disease is not sexually transmitted.

Affected Populations

In the USA, bejel remains a rare disorder. It was brought to the USA via immigration of families from countries in which it is common (endemic), or by Americans who may have contracted the disease while in a hot, dry country.

Bejel exhibits no preferences or propensities as to gender or race. Children living in unsanitary conditions are most often affected. About 25% of cases involve children of six years or younger; about 55% involve children and adolescents of age 16 years or younger, while about 20% involve persons of adult age, especially those who are closely involved with children.

Related Disorders

Symptoms of the following disorders can be similar to those of Bejel. Comparisons may be useful for a differential diagnosis:

Acquired syphilis is a chronic infectious disease caused by the bacteria Treponema pallidum. It is transmitted by direct contact with an infected individual, usually through sexual intercourse. When left untreated, the symptoms of syphilis progress (i.e., primary, secondary, and latent stages). Eventually any tissue or organ in the body may be affected. Early symptoms include lesions (chancres) of the skin, anus, vagina, or the moist surfaces of the mouth. The symptoms may remain dormant for years. (For more information on this disorder, choose "Syphilis" as your search term in the Rare Disease Database.)

Pinta is a rare infectious tropical disease affecting the skin that is caused by the bacterium Treponema Carateum, which is transmitted by direct, nonsexual contact. Pinta progresses through three distinct stages, which are characterized by various skin lesions and discoloration. Other organ systems are not affected. Exposed areas of the skin such as the face and extremities are most often affected. (For more information on this disorder, choose "Pinta" as your search term in the Rare Disease Database.)

Yaws is an infectious tropical disease caused by the spirochete (spiral shaped) bacterium known as Treponema pertenue. The disease presents in three stages of which the first and second are easily treated. The third, however, may involve complex changes to the bones in many parts of the body. The first stage is characterized by the appearance of small, painless bumps on the skin that group together and grow until they resemble a strawberry. The skin may break open, forming an ulcer. The second stage (usually starting several weeks or months after the first) presents with a crispy, crunchy rash that may cover arms, legs, buttocks and/or face. If the bottoms of the feet are involved, walking is painful and the stage is known as "crab yaws." Stage 3 yaws involves the long bones, joints, and/or skin. Yaws is very common in tropical areas of the world but rare in the United States. It is not a sexually transmitted disease. (For more information on this disorder, choose "yaws" as your search term in the Rare Disease Database.)

Standard Therapies

Diagnosis The diagnosis of bejel is based on the geographic history of the patient as well as laboratory testing of material from the lesions (darkfield examination). The responsible bacterium is readily identifiable on sight in a microscope as a treponema. Samples of the fluid that surrounds the lesions yield a positive VDRL (Venereal Disease Research Laboratory) and FTA-ABS (fluorescent treponemal antibody-absorption) test. However, additional history may be required to differentiate bejel from venereal syphilis.

Treatment The standard treatment for bejel is the antimicrobial drug benzathine penicillin G. Those individuals who are allergic to penicillin may be given doxycycline or tetracycline. The lesions caused by this disease typically heal after treatment. However, scarring may be permanent.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Research on tropical diseases is ongoing. The development of a treponemal vaccine is also being investigated. For more information about these disorders contact the World Health Organization (WHO) listed in the Resources section below.

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

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